Biliary atresia is a rare birth defect, affecting newborn infants. Biliary atresia blocks the bile ducts from the liver to the gallbladder. This blockage occurs when the bile ducts do not fully develop. This lack of development is a very serious condition. The bile ducts help to remove waste from the liver and carry salts that help the small intestine digest fat. When the bile ducts are blocked, it can lead to potentially fatal conditions such as liver damage and cirrhosis of the liver.
Symptoms of Biliary Atresia
Infants with biliary atresia appear to be normal at birth. Symptoms start to develop by the time they reach 2 to 3 weeks old. Jaundice, yellowing of the skin and eyes, may develop as the first symptom at around 2 to 3 weeks, while weight loss and irritability might appear after the first month. Some babies with biliary atresia may develop jaundice at birth while others may develop it over several months. It might either get worse or come and go. Other symptoms to look for would be dark urine, slow growth in the child, enlarged spleen, or pale or clay coloured, foul smelling stools. If you notice any of these symptoms with your infant, it is important to contact your child’s doctor as soon as possible.
Diagnosis of Biliary Atresia
If the paediatrician suspects your child has biliary atresia, your child will be referred to a specialist to treat the condition. Specialists who treat biliary atresia include a paediatric hepatologist, who is an expert in liver disease of children, a paediatric gastroenterologist, who is an expert in digestive diseases of children and a paediatric surgeon.
Initial tests for biliary atresia include a physical exam where the physician presses on the baby’s abdomen to check for an enlarged liver. Other tests include blood, stool and urine samples to check for liver disease. In the blood test, the level of bilirubin is measured, as well as other tests for other problems. Other tests include abdominal x-rays and ultrasound, liver scans such as Hepatobiliary iminodiacetic acid (HIDA) scan, an x-ray of the bile ducts and a liver biopsy.
Abdominal X-rays and ultrasounds cannot be used to make the diagnosis of biliary atresia, although they can help to rule out other common causes of the jaundice. The x-ray and ultrasound can also show whether the liver or bile ducts are enlarged and whether tumours or cysts are obstructing the bile ducts. Liver scans such as the (HIDA) scan, can detect a blockage of bile ducts.
For a liver scan, the baby will be sedated while a needle is injected through the skin and then quickly in and out of the liver. A small piece of liver is extracted from the infant for microscopic examination. This examination will not only rule out other liver conditions, but can also usually show whether biliary atresia is present.
Treatment of Biliary Atresia
The only treatment for biliary atresia is surgery. For the surgery, the baby will be under general anaesthetic while the surgeon makes an incision, to examine the bile ducts and the liver. If the diagnosis of biliary atresia is confirmed, one of the surgical options, called the Kasai procedure, is performed while the baby is still asleep. The Kasai procedure involves removing the bile ducts, and bringing up a loop of small intestine to drain the liver. This results in the bile flowing from the small bile ducts straight into the intestine. If the Kasai procedure is unsuccessful, then a liver transplant is necessary. If done within the first 1 or 2 years, the liver transplant increases the chances of the infant’s survival.